World Federation of Hemophilia Report on the · 2010-12-13 · regulatory agency and cannot make recommendations relating to safety of manufacturing of specific blood products. For

DECEMBER 2007

World Federation of Hemophilia Report on the

ANNUAL GLOBAL SURVEY 2006

Treatment for All

WFH Global Survey 2006

Report on the Annual Global Survey 2006 is published by the World Federation of Hemophilia. All data are provisional. © World Federation of Hemophilia, 2007 Permission to reproduce or translate this document in whole or in part, is granted to affiliated hemophilia organizations and hemophilia treatment centres, with appropriate acknowledgement of the WFH. However, permission does not extend to the reproduction or translation of this document, in whole or in part, for sale or for use in conjunction with commercial purposes. To obtain permission to reprint, redistribute, or translate this publication, please contact the Communications Department at the address below. Please note: This material is intended for general information only. The World Federation of Hemophilia does not endorse particular treatment products or manufacturers; any reference to a product name is not an endorsement by the World Federation of Hemophilia. The WFH is not a regulatory agency and cannot make recommendations relating to safety of manufacturing of specific blood products. For recommendations of a particular product, the regulatory authority in a particular country must make these judgments based on domestic legislation, national health policies and clinical best-practices. World Federation of Hemophilia 1425 René Lévesque Boulevard West, Suite 1010 Montreal, Quebec H3G 1T7 Tel. (514) 875-7944 Fax: (514) 875-8916 E-mail: [email protected] Website: www.wfh.org


Table of Contents Introduction……………………………………….…………… 1

Summary of survey data……………………………………. 3

Countries included in the survey…………………………… 4

Population statistics …………………………………..….…. 5

Demographic details ………………………………………… 8

Patients with inhibitors ……………………………………… 12

HIV and HCV infection ……………………………………… 14

Reported use of treatment products .………..……………. 16

Healthcare system…………………………………….……… 19

Use of factor concentrates…………………….……….……. 22

Factor use per capita…………………………………….…… 25

Annual Global Survey 2006 sample questionnaire……….. 26

Glossary………………………………………………………… 31

World Federation of Hemophilia Global Survey 2006



1 WFH Global Survey 2006

Introduction to the Report on the WFH Global Survey 2006

Report on the Annual Global Survey 2006 includes selected demographic and other data on people with hemophilia (PWH), von Willebrand disease (vWD), other rare bleeding disorders, and platelet disorders throughout the world. The purpose of this report is to provide useful information to hemophilia organizations, hemophilia treatment centres (HTCs), and health officials involved in efforts to reduce or prevent complications of bleeding disorders in order to assist with program planning. Methodology In 1998, the World Federation of Hemophilia (WFH) began collecting information on hemophilia care throughout the world. This survey, called the WFH Global Survey, collects basic demographic information, data on resources of care and treatment products, and information on the prevalence (the percentage of the population affected) of infectious complications such as HIV and hepatitis C (HCV). The WFH compiled the first survey report in 1999. Each year questionnaires are sent to national hemophilia associations linked with the WFH with the request that they in turn work with physicians or health officials, as necessary, to complete the survey. The WFH reviews completed questionnaires for inconsistencies, which are clarified where possible by communicating directly with the participating organization. The 2006 survey is the eighth WFH survey. It gathers data for the years 2004, 2005 and 2006. The survey includes data on more than 200,000 people with hemophilia, von Willebrand disease and other bleeding disorders in 100 countries. Data from the WFH questionnaire are supplemented with data from other sources in order to provide a general socio-economic picture of each country surveyed. Comments on data collection Participation in the survey is voluntary. Although these data are self-reported, fairly consistent information on hemophilia care has been obtained from countries with similar economic capacities, validating its use for program planning. This year national hemophilia organizations have supplied more complete data than last year, including information on inhibitors and more detailed numbers on rare bleeding disorders. Answers are not always available for all questions. In such cases, the analysis is done using only data from countries that responded, with the number of respondents as the denominator. This report provides information on the use of treatment products. It includes only those countries where the national hemophilia organization provided information. Quantities reported used were not independently verified.


WFH Global Survey 2006 2



2006 WFH Global Survey Summary Demographics Number of countries in this survey: 100 Percentage of world population covered by 2006 survey: 88% Number of people identified with hemophilia A and B (question B1) : 137,352 Number of people identified with vWD (question B2): 48,276 Number of people identified with other bleeding disorders (question B3): 19,844 Total number of people with bleeding disorders identified: 205,472 Number of people with hemophilia A (question B10): 105,385 Number of people with hemophilia B (question B11): 21,408 Number of countries using national registries to report these numbers: 57 Reported number of PWH infected with HIV: 9,611 Reported number of PWH infected with HCV: 26,819 Reported number of patients with VWD infected with HIV: 913 Reported number of patients with VWD infected with HCV: 1,248 Factor usage (69 countries reporting) Per capita factor VIII usage (GNP above US$10,000): 4.46 IU Per capita factor IX usage (GNP above US$10,000): 0.78 IU Per capita factor VIII usage (GNP between US$2000-US$10,000): 0.90 IU Per capita factor IX usage (GNP between US$2000-US$10,000): 0.20 IU Per capita factor VIII usage (GNP below US$2,000): 0.11 IU Per capita factor IX usage (GNP below US$2,000): 0.003 IU Total reported annual global consumption of factor VIII concentrates: 5,125,564,772 IU Total reported annual global consumption of factor IX concentrates: 917,694,557 IU



Countries included in the 2006 WFH Global Survey

1. Albania 2006 2. Algeria 2006 3. Argentina 2006 4. Armenia 2004 5. Australia 2006 6. Austria 2004 7. Azerbaijan 2005 8. Bangladesh 2006 9. Belarus 2006 10. Belgium 2006 11. Belize 2004 12. Bosnia-Herzegovina

2006 13. Brazil 2006 14. Bulgaria 2006 15. Canada 2006 16. Chile 2003 17. China 2006 18. Colombia 2006 19. Costa Rica 2005 20. Croatia 2006 21. Cuba 2006 22. Cyprus 2004 23. Denmark 2005 24. Dominican Republic

2006 25. Ecuador 2006 26. Egypt 2006 27. El Salvador 2004 28. Eritrea 2006 29. Estonia 2003 30. Finland 2006 31. France 2006 32. Georgia 2005

33. Germany 2006 34. Greece 2006 35. Guatemala 2005 36. Honduras 2006 37. Hungary 2006 38. Iceland 2005 39. India 2006 40. Indonesia 2006 41. Iran 2006 42. Iraq 2006 43. Ireland 2006 44. Israel 2005 45. Italy 2006 46. Jamaica 2004 47. Japan 2006 48. Jordan 2006 49. Kenya 2006 50. Korea 2006 51. Kuwait 2005 52. Latvia 2006 53. Lebanon 2004 54. Lesotho 2006 55. Lithuania 2006 56. Macedonia 2004 57. Malaysia 2006 58. Malta 2003 59. Mexico 2006 60. Moldova 2005 61. Mongolia 2006 62. Nepal 2006 63. Netherlands 2006 64. New Zealand 2006 65. Nicaragua 2006 66. Nigeria 2005

67. Norway 2006 68. Pakistan 2006 69. Palestine 2006 70. Panama 2006 71. Peru 2006 72. Philippines 2006 73. Poland 2006 74. Portugal 2006 75. Qatar 2006 76. Romania 2006 77. Russia 2006 78. Saudi Arabia 2004 79. Senegal 2006 80. Serbia 2006 81. Singapore 2006 82. Slovak Republic 2006 83. Slovenia 2004 84. South Africa 2006 85. Spain 2006 86. Sri Lanka 2006 87. Sudan 2006 88. Sweden 2005 89. Switzerland 2006 90. Thailand 2006 91. Tunisia 2006 92. Turkey 2006 93. Ukraine 2006 94. United Kingdom 2006 95. United States 2006 96. Uruguay 2004 97. Uzbekistan 2006 98. Venezuela 2006 99. Viet Nam 2006 100.Zimbabwe 2006



Population Statistics

Country B1. # of PWH B2. # people with vWD

B3. # people with OBD

B4. Source of data

Albania 270 2 Registry

Algeria 1,145 107 148 Other

Argentina 1,933 339 Registry

Armenia 162 2 Survey

Australia 1,708 1,224 249 Registry

Austria 456 30 Survey

Azerbaijan 800 200 4 Registry

Bangladesh 333 6 Registry

Belarus 559 Other

Belgium 831 850 115 Other

Belize 14 3 1 Registry

Bosnia-Herzegovina 140 30

Brazil 8,169 2,316 333 Other

Bulgaria 572 69 27 Registry

Canada 2,921 2,564 870 Registry

Chile 1,027 Registry

China 5,044 Survey

Colombia 1,520 127 135 Registry

Costa Rica 167 Registry

Croatia 454 236 138 Registry

Cuba 364 248 Registry

Cyprus 103 12 5 Other

Denmark 445 334 109 Survey

Dominican Republic 317 58 61 Survey

Ecuador 356 42 12 Registry

Egypt 4,135 396 801 Registry

El Salvador 254 15 7 Other

Eritrea 36 Registry

Estonia 39 33 3

Finland 298 1,700 Registry

France 4,026 537 185 Survey

Georgia 218 12 12 Registry

Germany 5,000 3,100

Greece 841 712 142 Survey

Guatemala 123 14 72 Survey

Honduras 194 2 5 Survey

Hungary 976 1,303 467 Registry





B4. Source of data

Iceland 59 53 12 Registry

India 12,800 Registry

Indonesia 1,130 27 2 Registry

Iran 4,823 720 1,298 Registry

Iraq 700 135 137

Ireland 577 616 310 Registry

Israel 473 Survey

Italy 3,270 1,650 700 Registry

Jamaica 108 16 13 Registry

Japan 4,942 805 380 Survey

Jordan 248 45 Registry

Kenya 452 17 5 Other

Korea 1,724 94 55 Registry

Kuwait

Latvia 123 68 1 Survey

Lebanon 110 38 16 Other

Lesotho 16 2 Survey

Lithuania 149 120 30 Registry

Macedonia 208 18 Survey

Malaysia 1,034 355 324 Registry

Malta 15 4

Mexico 3,351 87 Other

Moldova 194 Survey

Mongolia 40 1 Registry

Nepal 195 Registry

Netherlands 1,600 Registry

New Zealand 530 143 25 Other

Nicaragua 227 37 5 Survey

Nigeria 42 3 4 Other

Norway 379 823 41 Registry

Pakistan 1,450 37 50 Survey

Palestine 132 7 50 Registry

Panama 243 286 14 Registry

Peru 509 90 14 Registry

Philippines 954 26 Registry

Poland 2,423 929 319 Registry

Portugal 639 48 14 Other

Qatar 112 7 62 Registry

Romania 1,500 281 50 Registry





B4. Source of data

Russia 5,056 1,196 930 Registry

Saudi Arabia 170 42 Other

Senegal 104 4 3 Survey

Serbia 451 208 14 Registry

Singapore 204 58 85 Registry

Slovak Republic 519 367 599 Registry

Slovenia 175 63 73 Registry

South Africa 1,689 503 208 Registry

Spain 1,920 683 289 Registry

Sri Lanka 231 4 Other

Sudan 359 23 27 Registry

Sweden 731

Switzerland 622 106 53 Registry

Thailand 1,244 68 46 Registry

Tunisia 270 65 Survey

Turkey 2,763 206 359 Registry

Ukraine

United Kingdom 7,429 8,583 6,651 Registry

United States 15,575 11,496 1,610 Survey

Uruguay 199 Registry

Uzbekistan 1,138 86 33 Registry

Venezuela 1,723 578 581 Registry

Viet Nam 1,047 30 146 Other

Zimbabwe 302 1 Registry

TOTALS 137,352 48,276 19,844



Demographic Details B10. Hemophilia A B11. Hemophilia B B12. Hemophilia, type unknown B13. von Willebrand disease B14a. Other hereditary bleeding disorders: Factor I deficiency B14b. Other hereditary bleeding disorders: Factor II deficiency B14c. Other hereditary bleeding disorders: Factor V deficiency B14d. Other hereditary bleeding disorders: Factor V+VIII deficiency B14e. Other hereditary bleeding disorders: Factor VII deficiency B14f. Other hereditary bleeding disorders: Factor X deficiency B14g. Other hereditary bleeding disorders: Factor XI deficiency B14h. Other hereditary bleeding disorders: Factor XIII deficiency B14i.1 Platelet disorders: Glanzmann's thrombasthenia B14i.2 Platelet disorders: Bernard Soulier Syndrome B14i.3 Platelet disorders: other or unknown B14j. Other hereditary bleeding disorders: type unknown

Country B10

. Hem

oph

ilia

A

B11

. Hem

oph

ilia

B

B12

. Hem

oph

ilia

type

un

know

n

B13

. von

Will

ebr

and

dise

ase

B14

a. F

acto

r I

defic

ienc

y B

14b

. Fac

tor

II de

ficie

ncy

B14

c. F

acto

r V

de

ficie

ncy

B14

d. F

acto

r V

+ V

III

defic

ienc

y

B14

e. F

acto

r V

II de

ficie

ncy

B14

f. F

acto

r X

de

ficie

ncy

B14

g. F

acto

r X

I de

ficie

ncy

B14

h. F

acto

r X

III

defic

ienc

y B

14i.1

Gla

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bast

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ia

B14

i.2 B

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Sou

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Syn

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B14

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let

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the

r

B14

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itary

bl

eed

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dis

ord

ers

Albania 240 30 2 2

Algeria 936 191 16 113 31 2 14 5 36 9 7 2 23 7

Argentina 1,700 233 178 339

Armenia 157 5 2

Australia 1,380 328 0 1,224 0 0 6 0 26 6 81 11 92

Austria 350 53 53 30

Azerbaijan

Bangladesh 289 44 1 5

Belarus 463 96

Belgium

Belize 11 3 3 1

Bosnia-Herzegovina 100 10 30

Brazil 6,887 1,282 206 2,316 38 12 99 20 34 18 82

Bulgaria 507 65 2 69 10 1 10 3 1

Canada 2,349 572 0 2,564 71 6 30 6 153 26 211 41 0 34 20 272

Chile 897 129 1

China

Colombia 1,161 247 112 127 17 9 2 8 13 1 8 4 15 3 23

Costa Rica 139 28 2 1 2 3

Croatia 374 80 0 11 11 0 8 23 10 5 10 7 37 0 0 1



Country B10

. Hem

oph

ilia

A

B11

. Hem

oph

ilia

B

B12

. Hem

oph

ilia

type

un

know

n

B13

. von

Will

ebr

and

dise

ase

B14

a. F

acto

r I

defic

ienc

y B

14b

. Fac

tor

II de

ficie

ncy

B14

c. F

acto

r V

de

ficie

ncy

B14

d. F

acto

r V

+ V

III

defic

ienc

y

B14

e. F

acto

r V

II de

ficie

ncy

B14

f. F

acto

r X

de

ficie

ncy

B14

g. F

acto

r X

I de

ficie

ncy

B14

h. F

acto

r X

III

defic

ienc

y B

14i.1

Gla

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B14

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Sou

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Syn

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dis

ord

ers

Cuba 303 61 2 0 0 1 0 0 2 1 0 0

Cyprus 52 61 12 5

Denmark 352 93 0 334 0 1 2 0 35 7 6 9 22

Dominican Republic 58 0 1 12 1

Ecuador 322 34 23 42 1 3 1 5 1

Egypt 3,224 801 396 100 134 6 60 68 78 11 23 2 298 24

El Salvador 229 25 15 5

Eritrea 31 5

Estonia 36 3 33 3

Finland 234 64 0 1,700 14 1 5 0 4 4 2 13

France 3,302 724 0 537 24 0 22 4 52 11 54 18 0

Georgia 186 35 0 12 1 12

Germany 4,055 745 1,200 3,100

Greece 714 127 712 8 3 1 33 4 25 10 18 14 26

Guatemala 114 9 14 72

Honduras 179 15 2 2 3

Hungary 789 187 1,303 14 5 16 166 18 61 3 7 2 28

Iceland 57 2 0 53 12

India 10,112 1,792 896

Indonesia 220 31 879

Iran 3,980 843 720 70 13 64 124 195 91 50 124 136 14 327

Iraq 525 175 135 26 1 34 5 1 5 65

Ireland 385 192 616 34 35 54 65 3 41 3 2 73

Israel 372 71 30

Italy 2,697 573 21 1,650 49 14 73 23 308 54 156 21 115 45

Jamaica 101 8 0 16 12

Japan 4,051 891 805 54 5 19 6 43 15 21 50

Jordan 202 46 1 51 3 3 6

Kenya 387 43 5 17

Korea 1,423 301 2 94 4 2 6 16 11 3 11

Kuwait

Latvia 103 20 68 1

Lebanon 88 22 38 16

Lesotho 16 0 2

Lithuania 128 21 0 120 0 0 2 0 21 1 1 3 0 0 0 10



Country B10

. Hem

oph

ilia

A

B11

. Hem

oph

ilia

B

B12

. Hem

oph

ilia

type

un

know

n

B13

. von

Will

ebr

and

dise

ase

B14

a. F

acto

r I

defic

ienc

y B

14b

. Fac

tor

II de

ficie

ncy

B14

c. F

acto

r V

de

ficie

ncy

B14

d. F

acto

r V

+ V

III

defic

ienc

y

B14

e. F

acto

r V

II de

ficie

ncy

B14

f. F

acto

r X

de

ficie

ncy

B14

g. F

acto

r X

I de

ficie

ncy

B14

h. F

acto

r X

III

defic

ienc

y B

14i.1

Gla

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s th

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i.2 B

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ord

ers

Macedonia 149 59 18

Malaysia 877 157 355 4 2 13 50 12 26 11 36 70

Malta 12 3 4

Mexico 2,902 449 274 87

Moldova

Mongolia 35 5 0 1

Nepal 165 30 1 1 3 1

Netherlands 1,500 100

New Zealand 446 84 143 25

Nicaragua 205 22 37 4 1

Nigeria 32 5 5 3

Norway 283 96 823 3 0 4 25

Pakistan 1,320 130 37

Palestine 105 27 9 12 3 4 26 1 48

Panama 218 25 286 2 10 1 1

Peru 439 73 75 91 0 0 1 0 4 1 5 1 0 2 0 0

Philippines 788 123 43 26

Poland 2,069 354 929 155

Portugal 498 103 38 48 2 3 6 1 2

Qatar 87 25 7 2 6 1 6 2 20 1

Romania 1,330 170 281 2 2 5 41

Russia 4,381 675 1,196

Saudi Arabia 129 38 3 42

Senegal 99 5 4 2 1

Serbia 388 63 208 1 1 4 4 3 1

Singapore 176 28 0 58 0 0 15 0 9 0 46 3

Slovak Republic 449 70 367 46 0 32 2 462 9 21 3 10 6 11

Slovenia 159 16 0 63 73

South Africa 1,426 263 503 9 0 41 4 18 10 30 6 8 11 23 48

Spain 1,645 275 683 15 3 14 3 22 8 24 20 6 16 158

Sri Lanka 4

Sudan 332 27 23 3 3 2 3 6 7

Sweden

Switzerland 516 106 0 53 9 0 0 4 20 3 3 14 0

Thailand 1,102 142 68 11 33



Country B10

. Hem

oph

ilia

A

B11

. Hem

oph

ilia

B

B12

. Hem

oph

ilia

type

un

know

n

B13

. von

Will

ebr

and

dise

ase

B14

a. F

acto

r I

defic

ienc

y B

14b

. Fac

tor

II de

ficie

ncy

B14

c. F

acto

r V

de

ficie

ncy

B14

d. F

acto

r V

+ V

III

defic

ienc

y

B14

e. F

acto

r V

II de

ficie

ncy

B14

f. F

acto

r X

de

ficie

ncy

B14

g. F

acto

r X

I de

ficie

ncy

B14

h. F

acto

r X

III

defic

ienc

y B

14i.1

Gla

nzm

ann'

s th

rom

bast

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ia

B14

i.2 B

erna

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Sou

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bl

eed

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dis

ord

ers

Tunisia 219 51 62 26 3 25 5 12 138 4

Turkey 2,276 487 206 1 6 22 77 39 6 2 8 10 2 32

Ukraine

United Kingdom 6,137 1,292 8,583 262 13 119 24 532 184 1,612 59 93 45 1,060

United States 11,900 3,675 11,496 83 52 162 559 89 552 113

Uruguay 152 9

Uzbekistan 1,022 116 86 7 3 33 5 1

Venezuela 1,342 381 578 48 11 17 21 72 78 128 8 15 6 5

Viet Nam 864 183 0 30 0 2 2 15 2 7 1 1 8 23 0 85

Zimbabwe 302 50 1

Total 105,385 21,408 4,063 46,927 1,037 148 917 328 3,476 886 3,379 635 815 296 1,423 1,774



Patients with clinically identified inhibitors

Country Hemophila A

Total patients

Hemophila A Patients with

clinically identified inhibitors

Hemophila B Total patients

Hemophila BPatients with


Algeria 936 24 191 1

Argentina 1,700 129 233 5

Australia 1,380 48 328 3

Belarus 463 29 96

Bulgaria 507 29 65

Canada 2,349 79 572 3

Colombia 1,161 227 247 23

Croatia 374 18 80 1

Egypt 3,224 10 801

France 3,302 71 724 2

Germany 4,055 104 745 14

Greece 714 21 127 2

Honduras 179 1 15

Hungary 789 22 187 1

Indonesia 220 50 31

Iran 3,980 170 843 1

Iraq 525 5 175

Ireland 385 8 192 1

Italy 2,697 288 573 8

Jordan 202 18 46 0

Korea 1,423 66 301 9

Latvia 103 2 20 1

Lesotho 16 1 0

Lithuania 128 8 21 0

Malaysia 877 53 157 3

Mexico 2,902 86 449 2

New Zealand 446 39 84 0

Nicaragua 205 5 22

Norway 283 0 96 9

Palestine 105 1 27

Panama 218 3 25 0

Peru 439 2 73 0

Philippines 788 11 123 1

Poland 2,069 152 354 3

Russia 4,381 122 675 2



Country Hemophila A

Total patients

Hemophila A Patients with


Hemophila B Total patients

Hemophila BPatients with


Serbia 388 14 63 0

Singapore 176 10 28 0

Slovak Republic 449 16 70 1

South Africa 1,426 442 263 62

Spain 1,645 57 275 5

Sudan 332 2 27

Switzerland 516 19 106 1

Thailand 1,102 35 142 1

Tunisia 219 6 51 0

Turkey 2,276 183 487 32

United Kingdom 6,137 172 1,292 8

United States 11,900 743 3,675 76

Uzbekistan 1,022 34 116

Venezuela 1,342 225 381 8

Viet Nam 864 7 183 0

Zimbabwe 302 2 50

TOTAL: 73621 3869 15907 289



HIV and HCV infection

Country

B1. Total people with hemophilia

B26. Hemophilia HIV infection

B27. Hemophilia HCV infection

B2. Total people with vWD

B28. vWD HIV infection

B29. vWD HCV infection

Albania 270 1

Algeria 1,145 10 143 107

Argentina 1,933 75 657 339

Australia 1,708 107 668 1,224 4 72

Austria 456 55 102 30 1

Azerbaijan 800 0 24 200

Bangladesh 333 2 1

Belize 14 0 0 3 0 0

Brazil 8,169 379 2,165 2,316

Bulgaria 572 9 516 69 0 50

Canada 2,921 219 837 2,564 6 67

Colombia 1,520 15 80 127 0 3

Costa Rica 167 19 62

Croatia 454 6 180 236 0 50

Cuba 364 4 125

Cyprus 103 2 13 12

Denmark 445 30 87 334 0 1

Dominican Republic 317 1 58

El Salvador 254 2 1 15

Estonia 39 0 21 33 0 4

Finland 298 2 137 1,700

France 4,026 418 1,510 537 11 103

Georgia 218 0 58 12 0 0

Germany 5,000 440 3,000 3,100

Greece 841 72 365 712 2 33

Guatemala 123 0 26 14 0 0

Hungary 976 10 302 1,303 2 100

Iceland 59 0 9 53

Indonesia 1,130 4 65 27

Iran 4,823 200 2,300 720 0

Iraq 700 182 135 24

Ireland 577 39 217 616 8

Israel 473 27 170

Italy 3,270 220 1,156 1,650 7 69

Jamaica 108 8 36 16 1 1

Japan 4,942 1,411 805 8

Korea 1,724 21 616 94 0 4



Country

B1. Total people with hemophilia

B26. Hemophilia HIV infection

B27. Hemophilia HCV infection

B2. Total people with vWD

B28. vWD HIV infection

B29. vWD HCV infection

Latvia 123 60 68 18

Lebanon 110 2 20 38 0 0

Lesotho 16 0 2 0 0

Lithuania 149 0 38 120 0 2

Macedonia 208 0 24 18 0 1

Malaysia 1,034 12 136 355 1

Mexico 3,351 30 98 87 1 2

Mongolia 40 0 0 1

Nepal 195 5

Netherlands 1,600 156 1,000

New Zealand 530 4 151 143 0 9

Nicaragua 227 1 97 37

Pakistan 1,450 1 18 37 9

Palestine 132 0 4 7 0 0

Panama 243 17 21 286 0 0

Peru 509 9 45 90 3 15

Philippines 954 0 2 26 0 0

Poland 2,423 6 929

Romania 1,500 15 900 281

Saudi Arabia 170 34 81 42 0 3

Senegal 104 0 2 4 0 0

Serbia 451 86 122 208 4 11

Singapore 204 0 65 58 0 2

Slovak Republic 519 0 159 367 0 23

South Africa 1,689 48 125 503 0 1

Spain 1,920 572 942 683 31 131

Sudan 359 1 25 23

Thailand 1,244 9 49 68 1

Tunisia 270 16 0 65

United Kingdom 7,429 2,887 397 8,583 789 6

United States 15,575 1,808 5,679 11,496 24 402

Uruguay 199 2

Uzbekistan 1,138 2 292 86 5

Venezuela 1,723 87 320 578 9 24

Viet Nam 1,047 1 109 30 0 2

Zimbabwe 302 1

TOTAL 100,409 9,611 26,819 44,481 913 1,248



Reported Use of Treatment Products

Country

B17. Availablility: plasma

B18. Availablility: cryo

B19. Availablility: pd concentrate

B20. Availablility: rec

B21. Availablility: DDAVP

Albania Sometimes Sometimes Sometimes Rarely Rarely

Algeria sometimes Sometimes always never never

Argentina Never Rarely Always Sometimes rarely

Armenia Always Sometimes Rarely Never Never

Australia Never Never Rarely Always Sometimes

Austria Never Never Sometimes Sometimes Rarely

Azerbaijan Always Rarely Rarely Rarely Never

Bangladesh Always Rarely Sometimes Sometimes Never

Belarus Rarely Always Sometimes Never Never

Belgium Never Never Always Always Rarely

Belize Always Sometimes

Bosnia-Herzegovina

Always Sometimes Sometimes Never Never

Brazil rarely rarely always sometimes sometimes

Bulgaria Never Never Always Rarely Rarely

Canada Never Never Rarely Always Sometimes

Chile Always Always Sometimes Rarely Rarely

China Sometimes Sometimes Sometimes Rarely Rarely

Colombia Rarely Always Sometimes Sometimes

Costa Rica Sometimes Always Sometimes Always

Croatia Never Rarely always Always Always

Cuba Rarely Sometimes Sometimes Sometimes Rarely

Cyprus Always Rarely

Denmark Never Never Never Always Always

Dominican Republic

Rarely Never Sometimes Rarely Sometimes

Ecuador Always Always Sometimes Rarely never

Egypt Always Always Rarely Rarely Sometimes

El Salvador Sometimes Sometimes Rarely Rarely Never

Eritrea Never Never Never Sometimes Never

Estonia Never Never Always Never Never

Finland Never Never Always Always Rarely

France Never Never Sometimes Sometimes Sometimes

Georgia Rarely Rarely Always Rarely Rarely

Greece Never Never Rarely Always Sometimes

Guatemala Always Sometimes Rarely

Honduras Sometimes Rarely Always Rarely

Hungary Never Never Sometimes Sometimes Sometimes



Country






Iceland Never Never Never Always Sometimes

India Sometimes Sometimes always Rarely Rarely

Indonesia Always Always Sometimes Rarely Never

Iran Always Always Always Rarely Sometimes

Ireland Always Always

Israel Rarely Rarely Sometimes Sometimes Rarely

Italy Never Never Sometimes Sometimes Sometimes

Jamaica Sometimes Sometimes Sometimes Rarely Rarely

Japan Never Never Always Always Rarely

Jordan Sometimes Sometimes Always Rarely

Kenya Always Sometimes Rarely Sometimes never

Korea Never Never Always Always Always

Kuwait Never Never Always Never Never

Latvia Never Never Always Rarely Always

Lebanon Sometimes Sometimes Sometimes Rarely Rarely

Lesotho always never never

Lithuania Never Never Always Sometimes Sometimes

Macedonia Sometimes Always Sometimes Never Sometimes

Malaysia Never Never always never Sometimes

Malta Always

Mexico Always Always always never Sometimes

Moldova Rarely Always Sometimes Sometimes

Mongolia Always Always Never Never Never

Nepal Always Sometimes Never Sometimes Rarely

Netherlands Never Never Always Always Always

New Zealand Rarely Never Sometimes Sometimes Sometimes

Nicaragua Always Sometimes Rarely never never

Nigeria Rarely Rarely Rarely Rarely Rarely

Norway Never Never Always Always Always

Pakistan Sometimes Sometimes Always Sometimes Sometimes

Palestine Always Sometimes Always Sometimes Rarely

Panama Rarely Sometimes always Rarely Always

Peru Sometimes Sometimes Always Never Rarely

Philippines Always Always Sometimes Rarely Never

Poland Never Never always Never Rarely

Portugal Never Never Sometimes Sometimes Sometimes

Qatar Sometimes Sometimes Sometimes Always Sometimes

Romania Sometimes Sometimes Sometimes Rarely Rarely



Country






Russia Always Always Always Rarely Rarely

Saudi Arabia Never Never Always Never Sometimes

Senegal Always Sometimes Sometimes Sometimes Rarely

Serbia Rarely Rarely Always Sometimes Sometimes

Singapore Never Never Always Sometimes Never

Slovak Republic Never Never Always Never Never

South Africa Never Never always Rarely Sometimes

Spain Never Never Always Always Always

Sri Lanka Sometimes Sometimes Sometimes

Sudan Rarely Rarely Always Rarely Never

Sweden Never Never Sometimes Always Sometimes

Switzerland Never Never Sometimes Sometimes Sometimes

Thailand Always Always Sometimes Rarely Sometimes

Tunisia Never Sometimes always Rarely never

Turkey Always Never Always Rarely Always

Ukraine Sometimes Sometimes Rarely Never Rarely

United Kingdom Rarely Rarely Sometimes Sometimes Sometimes

United States Rarely Rarely Sometimes Always Always

Uruguay Sometimes Sometimes Sometimes Never Rarely

Uzbekistan Always Always Sometimes

Venezuela Never Never Sometimes Sometimes Sometimes

Viet Nam Always Always Sometimes Rarely Rarely

Zimbabwe Sometimes Sometimes never never



Healthcare System

Country Population C1.

# H

TC

s

C2.

# p

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e ca

red

for

at

HT

Cs

C3.

Dia

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tic

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atio

n

C27

. HIV

co

mpe

nsat

ion

Albania 3,130,000 1 150 Clinical symptoms no no

Algeria 32,854,000 11 1,145 Factor assay no no

Argentina 38,747,000 19 Factor assay no yes

Armenia 3,016,000 0 Clotting screening test no no

Australia 20,155,000 15 1,708 Factor assay no yes

Austria 8,189,000 20 250 Factor assay no yes

Azerbaijan 8,411,000 1 150 Factor assay no no

Bangladesh 141,822,000 3 339 Factor assay no no

Belarus 9,755,000 0 0 Not known no no

Belgium 10,419,000 7 Factor assay no yes

Belize 270,000 3 14 Factor assay no no

Bosnia-Herzegovina 3,907,000 Factor assay no no

Brazil 186,405,000 30 11,355 Factor assay no no

Bulgaria 7,726,000 6 500 Factor assay no yes

Canada 33,390,141 25 6,355 Factor assay yes yes

Chile 16,295,000 Factor assay no no

China 1,315,844,000 6 Factor assay no yes

Colombia 45,600,000 8 1,350 Factor assay no no

Costa Rica 4,327,000 1 131 Factor assay no yes

Croatia 4,551,000 4 450 Factor assay no no

Cuba 11,269,000 303 Factor assay no no

Cyprus 835,000 1 64 Factor assay no no

Denmark 5,431,000 2 888 Factor assay no yes

Dominican Republic 8,895,000 1 212 Factor assay no no

Ecuador 13,228,000 2 150 Factor assay no no

Egypt 74,033,000 10 Factor assay no no

El Salvador 6,881,000 3 274 Factor assay no no

Eritrea 4,401,000 1 Factor Assay no no

Estonia 1,330,000 3 39 Factor assay no no

Finland 5,249,000 1 Factor assay no no

France 60,496,000 40 4,018 Factor assay no yes

Georgia 4,474,000 3 242 Factor assay no no

Germany 82,689,000 no no

Greece 11,120,000 4 450 Factor assay no no

Guatemala 12,599,000 1 no yes




# H

TC

s

C2.

# p

eopl

e ca

red

for

at

HT

Cs

C3.

Dia

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tic

tech

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C21

. HC

V

com

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atio

n

C27

. HIV

co

mpe

nsat

ion

Honduras 7,205,000 2 145 Factor assay no no

Hungary 10,098,000 8 2,746 Factor assay yes yes

Iceland 295,000 1 30 Factor assay no no

India 1,103,371,000 Factor assay no no

Indonesia 222,781,000 9 1,130 Clotting screening no no

Iran 69,515,000 10 6,841 Factor assay yes yes

Iraq 28,807,000 1 972 no no

Ireland 4,109,086 4 600 Factor assay yes yes

Israel 6,725,000 3 473 Factor assay no no

Italy 58,093,000 52 5,620 Factor assay yes yes

Jamaica 2,651,000 3 109 Factor assay no yes

Japan 128,085,000 8 Factor assay no yes

Jordan 6053193 3 Factor Assay no no

Kenya 34,256,000 1 221 Clotting screening test no no

Korea 47,817,000 12 Factor assay no no

Kuwait 2,687,000 Factor assay no no

Latvia 2,307,000 2 120 Factor assay no no

Lebanon 3,577,000 4 110 Factor assay no no

Lesotho 3,607,899 1 16 Factor assay no no

Lithuania 3,431,000 4 Factor assay no no

Macedonia 2,034,000 1 200 Factor assay no no

Malaysia 25,347,000 36 20 Factor assay no no

Malta 402,000 Not known no no

Mexico 107,029,000 52 3,712 Clotting Screening test no no

Moldova 4,206,000 Factor assay no no

Mongolia 2,646,000 3 Factor assay no no

Nepal 27,133,000 6 160 Factor assay no no

Netherlands 16,299,000 11 1,600 All no yes

New Zealand 4,028,000 6 500 Factor Assay yes yes

Nicaragua 5,487,000 1 227 clotting screening no no

Nigeria 131,530,000 0 clinical symptoms no no

Norway 4,620,000 1 400 Factor assay yes yes

Pakistan 157,935,000 10 1,500 Clotting Screening test no no

Palestine 3,702,000 6 132 Factor assay no no

Panama 3,232,000 2 543 Factor assay no no




# H

TC

s

C2.

# p

eopl

e ca

red

for

at

HT

Cs

C3.

Dia

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tic

tech

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C21

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V

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atio

n

C27

. HIV

co

mpe

nsat

ion

Peru 27,968,000 10 586 Clotting screening test no no

Philippines 83,054,000 4 100 Factor assay no no

Poland 38,530,000 32 Factor assay no no

Portugal 10,495,000 7 Factor assay no yes

Qatar 840,290 2 50 all no no

Romania 21,711,000 9 2,033 Factor assay/screening no no

Russia 143,202,000 4 1,800 Factor assay no no

Saudi Arabia 24,573,000 1 170 Factor assay no no

Senegal 11,658,000 1 104 Factor assay no no

Serbia 10,503,000 7 300 Factor assay no no

Singapore 4,326,000 3 80 Factor assay no no

Slovak Republic 5,401,000 44 390 Factor assay no yes

Slovenia 19,920,000 1 311 Factor assay no no

South Africa 47,432,000 11 831 Factor assay no no

Spain 43,064,000 34 Factor assay yes yes

Sri Lanka 20,743,000 1 Clotting Screening test no no

Sudan 36,233,000 1 200 Factor assay no no

Sweden 9,041,000 3 Factor assay yes yes

Switzerland 7,252,000 10 678 Factor assay no yes

Thailand 64,233,000 33 1,244 Factor assay no no

Tunisia 9,673,600 4 150 Factor assay no no

Turkey 73,193,000 34 3,328 Factor assay no no

Ukraine 46,481,000 2 Factor assay no no

United Kingdom 59,668,000 88 22,781 Factor assay yes yes

United States 298,213,000 135 28,795 Factor assay no yes

Uruguay 3,463,000 14 179 Factor assay no no

Uzbekistan 26,593,000 1 537 all no no

Venezuela 26,749,000 16 2,000 Factor assay no no

Viet Nam 84,238,000 5 Factor assay no no

Zimbabwe 13,010,000 2 50 Factor assay no no



Reported Use of Factor Concentrates

Country D1A

. F

acto

r V

III

tota

l IU

s

D1B

. F

acto

r IX

to

tal

IUs

D2A

. P

lasm

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ved

fa

cto

r V

III

D2B

. Pla

sma-

der

ived

fac

tor

IX

D3A

. R

eco

mb

inan

t fa

cto

r V

III

D3B

. R

eco

mb

inan

t fa

cto

r IX

D4A

. H

um

anit

aria

n

Aid

fac

tor

VII

I

D4B

. H

um

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aria

n

aid

fac

tor

IX

Albania 800,000 300,000 700,000 200,000

Algeria 9,000,000 1,512,000

Armenia 30,000 30,000

Australia 93,000,000 15,300,000 26,000,000 5,800,000 6,700,000 9,500,000

Azerbaijan 416,810 416,810

Bangladesh 690,815 17,150 591,770 17,150 99,045 138,875 17,150

Belarus 2,795,000 535,000

Belgium 60,000,000 13,000,000 47,000,000

Belize 70,000 120,000 20,000 50,000

Bosnia-Herzegovina

2,800,000 150,000 2,800,000 150,000

Brazil 224,000,000 60,000,000 224,000,000 60,000,000

Bulgaria 9,180,000 300,000 9,000,000 300,000 180,000

Canada 143,000,000 38,000,000 8,000,000 4,285,000 130,201,000 32,146,000

Colombia 34,000,000 2,500,000 33,000,000 900,000 1,000,000 1,600,000

Costa Rica 6,063,750 3,055,500

Croatia 11,163,045 1,142,500 9,656,202 1,142,500 1,503,843

Cuba 262,500 25,000 262,500 25,000

Dominican Republic

308,366 20,000 205,366

Ecuador 152,887 85,000 152,887 85,000

Eritrea 149,250 149,250 149,250

Finland 23,330,500 2,819,750 16,059,250 1,096,500 7,271,250 1,723,250 0 0

France 310,054,750 51,499,000 258,555,750

Georgia 1,851,175 359,500 1,800,000 308,500 51,175 51,000

Germany 560,000,000 80,000,000

Greece 28,765,383 4,027,642 3,772,659 268,540 24,992,724 3,759,102

Honduras 3,188 50,400 34,200

Hungary 51,300,000 4,500,000 49,800,000 4,500,000 1,500,000

Iceland 2,200,500 20,000 2,200,500 20,000

India 6,279,875 781,245 4,782,375 781,245 1,497,500 1,700,341 195,525

Indonesia 3,372,800 3,360,000 30,660 32,150

Iran 90,000,000 15,000,000 90,000,000

Ireland 22,600,000 8,570,000 16,075,250 7,667,500

Italy 300,000,000 50,000,000 120,000,000 20,000,000 180,000,000 30,000,000



Country D1A

. F

acto

r V

III

tota

l IU

s

D1B

. F

acto

r IX

to

tal

IUs

D2A

. P

lasm

a-d

eri

ved

fa

cto

r V

III

D2B

. Pla

sma-

der

ived

fac

tor

IX

D3A

. R

eco

mb

inan

t fa

cto

r V

III

D3B

. R

eco

mb

inan

t fa

cto

r IX

D4A

. H

um

anit

aria

n

Aid

fac

tor

VII

I

D4B

. H

um

anit

aria

n

aid

fac

tor

IX

Japan 291,000,000 38,900,000 114,363,000 38,900,000 17,663,700

Jordan 1,675,000 200,000 50,400 14,700

Kenya 2,600 2,600 2,600

Korea 100,667,750 15,779,250 64,279,750 2,296,250 36,388,000 13,482,000

Latvia 3,534,000 311,500 3,534,000 3,111,500

Lebanon 2,348,420 729,600 1,408,000 729,600 868,420

Lesotho 366,500 366,500

Lithuania 3,774,100 514,650

Macedonia 900,000 50,000 900,000 50,000 500,000

Malaysia 10,250,000 5,000,000 10,250,000 5,000,000

Mexico 52,031,500 10,275,600 52,031,500 10,275,600 105,000 82,000

Moldova 108,000 108,000

Nepal 327,296 65,505

New Zealand 20,220,000 3,525,000 650,000 2,400,000 13,000,000 992,000

Nigeria 50,000 50,000 50,000

Pakistan 305,979 11,154 180,000 125,979

Palestine 2,000,000 400,000 2,000,000 400,000 80,000

Panama 2,775,322 87,500 2,774,250 87,500 1,072 1,072

Peru 3,000,000 40,000 3,000,000 400,000 52,154

Philippines 871,694 169,970 857,814 169,970 13,880 99,444 169,970

Poland 60,000,000 6,000,000 60,000,000 6,000,000

Portugal 31,725,000 5,229,000 15,304,500 4,724,000 16,422,750 505,000

Romania 8,300,000 5,000,000 25,000,000 5,000,000

Russia 230,000,000 27,006,852 224,127,000 27,006,852 5,873,000

Senegal 35,000 15,000 20,000 20,000

Serbia 8,872,750 400,000 8,872,750 400,000

Singapore 3,250,000 450,000 3,241,000 45,000 9,000

Slovak Republic

15,900,000 1,300,000 15,900,000 1,300,000

South Africa 28,559,400 3,005,000 28,559,400 3,005,000

Spain 161,929,688 27,070,313 56,670,705 9,479,295 105,245,595 17,604,405

Sudan 3,190,235 264,610 3,168,235 264,610 22,000 22,000

Sweden 61,249,000 12,598,000 6,026,000 7,993,000 55,223,000 4,605,000

Switzerland 27,218,750 5,128,200 8,620,500 489,000 18,598,250 4,639,200

Turkey 70,496,500 8,322,500

Ukraine 15,000,000 15,000,000 15,000,000



Country D1A

. F

acto

r V

III

tota

l IU

s

D1B

. F

acto

r IX

to

tal

IUs

D2A

. P

lasm

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eri

ved

fa

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III

D2B

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der

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fac

tor

IX

D3A

. R

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mb

inan

t fa

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III

D3B

. R

eco

mb

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t fa

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D4A

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um

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aria

n

Aid

fac

tor

VII

I

D4B

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anit

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aid

fac

tor

IX

United Kingdom

253,275,120 46,026,311 71,050,647 19,930,911 182,224,473 26,095,400

United States 1,600,000,000 400,000,000 more than 1 million units

for Katrina aftermath

Uruguay 4,145,250 430,000 1,000,000

Uzbekistan 49,500 49,500

Venezuela 52,034,500 5,070,000 51,984,500 5,070,000 50,000 50,000

Viet Nam 40,000 40,000

Zimbabwe 3,187,800 50,400 34,200



Factor Use Per Capita Country FVIII per cap FIX per cap

Albania 0.256 0.096

Algeria 0.274 0.046

Australia 4.614 0.759

Azerbaijan 0.050

Bangladesh 0.005 0.000

Belarus 0.287 0.055

Belgium 5.759

Belize 0.259 0.444

Bosnia-Herzegovina 0.717 0.038

Brazil 1.202 0.322

Bulgaria 1.188 0.039

Canada 4.283 1.138

Colombia 0.746 0.055

Costa Rica 1.401 0.706

Croatia 2.453 0.251

Dominican Republic 0.035 0.002

Ecuador 0.012 0.006

Eritrea 0.034

Finland 4.445 0.537

France 5.125 0.000

Georgia 0.414 0.080

Germany 6.772 0.967

Greece 2.587 0.362

Hungary 5.080 0.446

Iceland 7.459 0.068

Indonesia 0.015

Iran 1.295 0.216

Ireland 5.500 2.086

Italy 5.164 0.861

Japan 2.272 0.304

Jordan 0.277 0.033

Korea 2.105 0.330

Latvia 1.532 0.135

Country FVIII per cap FIX per cap

Lebanon 0.657 0.204

Lesotho 0.102

Lithuania 1.100

Macedonia 0.442 0.025

Malaysia 0.404 0.197

Mexico 0.486 0.096

Nepal 0.012 0.002

New Zealand 5.020 0.875

Pakistan 0.002 0.000

Palestine 0.540 0.108

Panama 0.859 0.027

Peru 0.107 0.001

Philippines 0.010

Poland 1.557 0.156

Portugal 3.023 0.498

Romania 0.382 0.230

Russia 1.606 0.189

Serbia 0.845 0.038

Singapore 0.751 0.104

Slovak Republic 2.944 0.241

South Africa 0.602 0.063

Spain 3.760 0.629

Sudan 0.088 0.007

Sweden 6.775 1.393

Switzerland 3.753 0.707

Turkey 0.963 0.114

Ukraine 0.323

United Kingdom 4.245 0.771

United States 5.365 1.341

Uruguay 1.197 0.124

Venezuela 1.945 0.190

Zimbabwe 0.245



Sample Survey Questionnaire A. National Hemophilia Organization A1. Organization name

A2. Address

A3. City

A4. State, Province, Region, Prefecture, County

A5. Postal/ZIP Code

A6. Country

A7. Phone

A8. Fax

A9. E-mail

A10. Website

Executives of your association

Function Name

A11. President/Chairman

A12. Medical Advisor

A13. Executive Director

A14. International Contact

A15. Number of full time staff working for the association

A16. Number of active members in your hemophilia association/society

A17. How many members in your association are medical doctors?

A18. Does your association have an annual meeting? Yes No

A19. If yes, how many people attended this meeting?

A20. Does your association produce an annual report? Yes No

B. Population Statistics

(Please DO NOT estimate or guess) Number Not known

B1. Number of identified people with hemophilia A and B (PWH)

B2. Number of identified people with von Willebrand disease (vWD)

B3. Number of identified people with other hereditary bleeding disorders (including rare factor deficiencies and inherited platelet disorders)

B4. What is the source of the numbers provided above? Check one

Hemophilia registry of PWH and other inherited bleeding disorders in your country

Survey of your country's hemophilia treatment centres

Other (Describe):



B5. Please assess what proportion of your country’s patient population is included in the numbers provided for questions B1-3.

0-25% 25-50% 50-75% 75-100%

Age distribution of people with Hemophilia and von Willebrand disease

Age group Number with hemophilia Number with vWD

B6. 0 - 13 years old

B7. 14 - 18 years old

B8. 19 years old and over

B9. No age data

Type of hereditary bleeding disorders

Diagnosis Number of persons

B10. Hemophilia A

B11. Hemophilia B

B12. Hemophilia, type unknown

B13. von Willebrand disease

B14a. Other hereditary bleeding disorders: Factor I deficiency

B14b. Other hereditary bleeding disorders: Factor II deficiency

B14c. Other hereditary bleeding disorders: Factor V deficiency

B14d. Other hereditary bleeding disorders: Factor V+VIII deficiency

B14e. Other hereditary bleeding disorders: Factor VII deficiency

B14f. Other hereditary bleeding disorders: Factor X deficiency

B14g. Other hereditary bleeding disorders: Factor XI deficiency

B14h. Other hereditary bleeding disorders: Factor XIII deficiency

B14j. Other hereditary bleeding disorders: type unknown

B14i.1 Platelet disorders: Glanzmann's thrombasthenia

B14i.2 Platelet disorders: Bernard Soulier Syndrome

B14i.3 Platelet disorders: other or unknown Number of identified people with hemophilia by diagnosis of severity

Type of hemophilia Mild Moderate Severe No Data

B15a. Hemophilia A

B16a. Hemophilia B

INHIBITORS: Number of identified people with hemophilia with current clinically significant inhibitors. (Patients who do not respond to standard treatment.)

Type of hemophilia Number with current inhibitors No Data

B15b. Hemophilia A

B16b. Hemophilia B

Products used to treat hemophilia

B17. Plasma always sometimes rarely never

B18. Cryoprecipitate always sometimes rarely never

B19. Plasma-derived concentrate always sometimes rarely never



B20. Recombinant always sometimes rarely never

B21. DDAVP (Desmopressin) always sometimes rarely never

Products used to treat vWD

B22. Plasma always sometimes rarely never

B23. Cryoprecipitate always sometimes rarely never

B24. Plasma-derived concentrate always sometimes rarely never

B25. DDAVP (Desmopressin) always sometimes rarely never

HIV and hepatitis C testing among living people with hemophilia

Infectious Disease Number of people tested Number of people infectedB26. HIV

B27. Hepatitis C

HIV and hepatitis C testing among living people with von Willebrand disease

Infectious Disease Number of people tested Number of people infectedB28. HIV

B29. Hepatitis C

Number and cause of deaths of people with bleeding disorders (January 1-December 31, 2006)

Cause of death Number of people with Hemophilia A & B

Number of people with von Willebrand disease

Number of people with other inherited bleeding disorders

B30. Bleeding

B31. HIV

B32. Liver disease

B33. Other causes C. Hemophilia Care System in Your Country

C1. How many hemophilia treatment centres are there in your country?

C2. Number of hemophilia patients regularly cared for by all these hemophilia treatment centres:

C3. How are the majority of people with hemophilia diagnosed? (check one only)

Clinical symptoms only

Clotting screening tests only (e.g. PT or APTT)

Specific factor assays for factor VIII or IX

Not known

C4. Who pays for the medical care for the majority of people with hemophilia? (check one only)

Government (social security, public health care system, etc.)

Private insurance

Patient

C5. Who pays for the medicine for the majority of people with hemophilia? (check one only)

Government (social security, public health care system etc.)

Private insurance

Patient



Infectious diseases testing and vaccination

HIV

C6. Are more than 50% of hemophilia A and B patients tested for HIV?

Yes

No

C7. Are more than 50% of people with vWD tested for HIV?

Hepatitis A

C8. Are more than 50% of people with hemophilia vaccinated against hepatitis A?

C9. Are more than 50% of people with vWD vaccinated against hepatitis A?

Hepatitis B

C10. Are more than 50% of people with hemophilia tested for hepatitis B?

C11. Have more than 50% of people with hemophilia been vaccinated against hepatitis B?

C12. Are more than 50% of people with vWD tested for hepatitis B?

C13. Have more than 50% of people with vWD been vaccinated against hepatitis B?

Hepatitis C

C14. Are more than 50% of people with hemophilia tested for hepatitis C?

C15. Are more than 50% of people with vWD tested for hepatitis C? Home Care

C16. What is the percentage of people with hemophilia using home care (infusing clotting factor concentrates at home)?

Not available Less than 10% Between 10% to 50% More than 50%

C17. What is the percentage of people with vWD using home care (home infusion)?

Not available Less than 10% Between 10% to 50% More than 50%

Treatment products

C18. Who pays for hemophilia treatment products in your country?

(Check any that apply)

If costs are shared, pleaseindicate the percentage paid by

each contributor Government/health ministry

Private insurance

Patients/NMO/charitable institutions

C19. Who is responsible for choosing hemophilia treatment products used in your country?

Government, health ministry or central pharmacy

Hospitals or doctors

C20. How is the hemophilia treatment product bought?



Central purchasing for the whole country

Purchase through individual distributor

Other (please describe) Compensation for HCV infection

C21. Has there been compensation for HCV infection due to blood products in your country?

If YES, please answer the following questions:

Yes

No

C22. Who paid the compensation? Government

Private companies

Other

C23 How much was paid to each individual that was compensated?

C24. What percentage of people with hemophilia infected with HCV were compensated?

C25. How is the compensation paid? One payment other (please describe)

C26. What years when patients were infected does the compensation apply to?

Start Finish

Compensation for HIV infection

C27. Has there been compensation for HIV infection due to blood products in your country?

If YES, please answer the following questions:

Yes

No

C28. Who paid the compensation? Government

Private companies

Other

C29 How much was paid to each individual that was compensated?

C30. What percentage of people with hemophilia infected with HIV were compensated?

C31. How is the compensation paid? One payment other (please describe)

C32. What years does the compensation apply to? Start Finish



D. The Cost and Use of Factor Concentrates

Annual usage of factor concentrates Factor VIII Factor IX Not known

D1. How many international units (IU) of factor concentrates were used in your country in 2006?

D2. How many international units of plasma-derived concentrates were used in your country in 2006?

D3. How many international units of recombinant concentrates were used in your country in 2006?

D4. How many international units were humanitarian aid?

Glossary of terms

Bernard-Soulier syndrome: A severe congenital bleeding disorder characterized by thrombocytopenia and large platelets, due to a defect in the platelet glycoprotein 1b/V/IX receptor .

Cryoprecipitate: A fraction of human blood prepared from fresh plasma. Cryoprecipitate is rich in factor VIII, von Willebrand factor and fibrinogen (factor I). It does not contain factor IX.

Desmopressin (DDAVP): A synthetic hormone used to treat most cases of von Willebrand disease and mild hemophilia A. It is administered intravenously by subcutaneous injection or by intranasal spray.

Factor concentrates: These are fractionated, freeze-dried preparations of individual clotting factors or groups of factors derived from donated blood.

Glanzmann's thrombasthenia: A severe congenital bleeding disorder in which the platelets lack glycoprotein Iib/IIIa, the blood platelet count is normal, but their function is very abnormal.

Hemophilia A: A condition resulting from factor VIII deficiency, also known as classical hemophilia.

Hemophilia B: A condition resulting from factor IX deficiency, also known as Christmas disease.

Hemophilia treatment centre: A specialized medical centre that provides diagnosis, treatment, and care for people with hemophilia and other inherited bleeding disorders.

HIV: Human immunodeficiency virus. The virus that causes AIDS

Home care: The patient administers treatment product himself/herself in his/her own home.

Identified person: A living person known to have hemophilia, von Willebrand disease, or another bleeding disorder.

Inhibitors: A PWH has inhibitors when their body's immune system attacks the molecules in factor concentrate, rendering it ineffective.

International Unit (IU): A standardized measurement of the amount of factor VIII or IX contained in a vial. Usually marked on vials as 250 IU, 500 IU, or 1000 IU.

Mild hemophilia: Condition resulting from a level of factor VIII or factor IX clotting activity between 6 to 24% of normal activity in the bloodstream.

Moderate hemophilia: Condition resulting from a level of factor VIII or factor IX clotting activity between 1 to 5 % of normal activity in the bloodstream.



Plasma-derived products: Factor concentrates that contain factor VIII or IX that have been fractionated from human blood.

PWH: Person with Hemophilia.

Recombinant products: Factor concentrates that contain factor VIII or IX that have been artificially produced and are, therefore, not derived from human blood.

Registry: A database or record of identified people with hemophilia or inherited bleeding disorders. A registry includes information on personal details, diagnosis, treatment and complications

Severe hemophilia: Condition resulting from a level of factor VIII or factor IX clotting activity of less than 1 % in the bloodstream.

von Willebrand disease: An inherited bleeding disorder resulting from a defect or deficiency of von Willebrand factor.

vWD: von Willebrand disease.

World Federation of Hemophilia 1425 René Lévesque Boulevard West, Suite 1010

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www.wfh.org

© World Federation of Hemophilia, 2007.

World Federation of Hemophilia Report on the · 2010-12-13 · regulatory agency and cannot make recommendations relating to safety of manufacturing of specific blood products. For

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